In eight, six, and one patient, the lumbosacral dysraphic lesion was a myelomeningocele, spinal lipoma, and limited dorsal myeloschisis, respectively. Four patients underwent initial surgery to repair the lesion or previously received untethering surgery at another hospital. The duration from the previous surgery ranged from one to 23 years (mean: 9.2 years). Symptoms before untethering were worsening of neurogenic bladder in six patients, deterioration of the moto-sensory function of the lower extremities in five patients, and progression of orthopedic symptoms (scoliosis and the ankle joint deformities) in four patients (Table 1).
Circumferential, complete untethering was achieved in all the patients. The BCR was monitorable in nine patients, was stable in six patients, and was worse but preserved in three patients. None of the patients, including those whose BCR was unmonitorable, experienced postoperative urological deterioration [9]. All but one patient reported improvement or stabilization of the preoperative symptoms during the follow-up period (range: 12–104 months: mean: 48.6 months) (Table 1).
Postoperative MRI demonstrated a ventrally anchored conus medullaris and restored dorsal subarachnoid space in 14 patients (Table 2). In only one patient (case 1), the first to undergo the procedure in the cohort and who showed no improvement after the untethering surgery, postoperative MRI demonstrated that the untethered conus medullaris had again come into contact with the dorsal dura mater. One patient (case 7) showed re-attachment of the conus medullaris to the dorsal dura (re-TSC) on MRI at postoperative year 1 (Fig. 2). In other patients (cases 12 and 14), the conus medullaris shifted dorsally, and the dorsal subarachnoid space became unclear on follow-up MRI at postoperative year 1. The remaining eleven patients had no dorsal re-tethering at the last follow-up MRI performed between postoperative years 1 to 7 (Fig. 3) (Table 2).
No complication directly related to the ventral anchoring was observed, and there was no TCS recurrence during the follow-up period. In addition, there was no case of the conus medullaris touching the ventral dura mater as a result of the ventral anchoring on the last follow-up MRI.
Representative cases
Case #8
The patient, a 37-year-old female, underwent repair for a myelomeningocele soon after birth and received a VP shunt for hydrocephalus. She underwent her first untethering surgery at age 14 years at another hospital after left knee pain developed. At presentation, she had a one-year history of pain in the left sole which slowly worsened and became recalcitrant to medical treatment. She was referred to our hospital for further treatment. Her motor functions were stable although she was paraparetic and required a wheelchair for daily activity. Sensory disturbance was present below the L4 level. MRI revealed a dorsal shift of the spinal cord and the caudal end tethered at L4 (Fig. 4). Marked lumbosacral lordosis associated with TSC was observed [10]. L4 partial laminectomy was performed intraoperatively. Following dural opening, the thick arachnoid membrane was dissected, and the TSC was completely untethered from the dura. The conus medullaris was circumferentially dissected from the dura mater. Then, ventral anchoring was performed between the scar tissue of the conus medullaris and the ventral dura mater. The dorsal dura mater was then closed directly. Her postoperative course was uneventful, and the left sole pain improved markedly. MRI at postoperative week 1 demonstrated the untethered conus medullaris in the middle of the subarachnoid space (Fig. 4).
Case #15
A 16-year-old female patient received surgery for a type 2 spinal lipoma (lipomyelomeningocele) at age 25 days [11]. Subsequently, she underwent two operations for TCS at age 4 years and 7 years at another hospital. Her left ankle joint deformity and weakness gradually deteriorated over the past several years, and sudden weakness developed in the left leg, causing walking difficulty several times a month. She was referred to the senior author (NM) for further treatment. MRI demonstrated a dorsal shift in the position of the spinal cord and a conus medullaris-residual spinal lipoma complex attached to the dorsal dura mater and forming a cystic lesion caudal to the subarachnoid space (Fig. 5). TCS recurrence was diagnosed, and the third untethering surgery was performed. L5 partial laminectomy was performed intraoperatively. Partial resection of the residual lipoma was carried out, and the caudal cyst wall was opened. The conus medullaris was circumferentially untethered from both the rostral and caudal sides. Then, ventral anchoring was performed in the previously described manner. The dura mater was then closed directly. Her postoperative course was uneventful, and the left leg weakness resolved soon after surgery. MRI at postoperative week 1 demonstrated the untethered conus medullaris with a restored dorsal subarachnoid space (Fig. 5).